Intrahepatic bile duct papillomatosis associated with changes of nuclear atypia is described in a young man. Radical excision was performed by left hepatic lobectomy and removal of the common bile duct and left hepatic ducts. Fourteen previous cases of intrahepatic or diffuse intrahepatic and extrahepatic biliary papillomatosis are reviewed. While local recurrence and progression to death from obstructive jaundice and cholangitis is the rule, only two definite cases of malignant change have been reported. Radical surgery may be curative for this rare condition.